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Have you ever felt grateful to have a healthy bladder system? Imagine if you have problems with it, you probably will have a difficult time passing urine comfortably. Although, at times we do wish the bladder can follow our own instruction especially when we are left with no choice when the public restroom is full or had to wait a long time for a toilet break. However, what happens if the bladder is not functioning normally, or even looks normal as many other people do? In this DoctorOnCall’s article, we will be learning about bladder exstrophy.

Before we understand more about bladder exstrophy, you might want to know a bit more about the urinary system in general. The urinary system is a function to filter blood and produce urine as the body waste. The urinary system helps to maintain balance fluid and salt or electrolytes such as potassium and sodium in blood. It also plays a role in maintaining blood pressure. It consists of kidney, ureter, bladder and urethra. A normal urine which is pale straw or transparent yellow colour indicates a person with a healthy urinary system.

The bladder is a triangle-shaped organ located in the lower abdomen. It is held by ligaments that are attached to other organs and the pelvic bones. The bladder relaxes and expands to store urine, and contract and flatten to empty urine through the urethra. Typical healthy adult bladder can store up to 500 mL of urine in women and 700 mL in men. The need to urinate can be felt as early when the bladder has between 200 to 350 mL urine. The bladder muscle tightens to squeeze urine out of the bladder while the sphincter muscle relaxes to allow urine exiting the bladder through the urethra.

Bladder exstrophy is a rare birth defect. It is a condition where the bladder and parts around it are flattened and exposed to the abdominal wall. This birth defect is seen in one out of 10 000 to 50 000 live births. In a family with a child with exstrophy, the likelihood of the second child to be born with exstrophy is 1 in 100. The risk for having a child with exstrophy is 1 in 70 if the parents have exstrophy.

The exact causes of exstrophy is not known. Even so, there is theory that supports the possible cause of exstrophy. Theories suggest that the normal structure known as cloacal membrane might overdevelop in babies. This might prevent the tissue development, ingrowth and joining together of the supportive lower abdominal wall structure. There are studies showing clustering conditions of exstrophy in families due to inherited factors.

Exstrophy is a serious condition. This is because the child would not be able to hold the urine. They will also be more at risk for sexual dysfunction and have increased risk for bladder cancer. This is why it is important for children with exstrophy to get treated as it can definitely give great impact to their future.

Treatment for exstrophy mainly focuses on surgery, particularly reconstruction surgery. The main goal of treatment is includes to  close the bladder, the back of the urethra and pelvis, to rebuild the penis to look normal and functional in boys or rebuild the outer sex organs in girls and to fix the bladder so that it is able to hold urine until it is time to urinate. The treatment usually involves separate operations at various times in the life of the child to obtain the best outcome.

The first operation is the initial closure by reforming the pelvic bones into the normal ring shape. This is also the stage where the bladder, the abdominal wall and the posterior urethra are closed and the belly button is reconstructed. After this surgery, the baby is placed with the lower legs in traction to prevent separating the pelvic bones. This surgery typically done after a few months of the baby birth although at times it may be done right after the baby is born. About 6 months after the bladder closure, surgery to rebuild the sex organ is done. When the bladder has grown into sufficient size and the child is ready for potty-training (around the age of 4 or 5), surgery to the bladder neck is done to achieve continence. Often, further operations are needed to improve the child’s ability to urinate or to improve the outer sex organs. In more complicated situations, longer-term management is needed.

It can be concluded that exstrophy is a rare condition and occurs even before birth. It is a birth defect that should be treated by a healthcare professional in a timely manner. It is important for parents to ensure their child is getting the right support and care when receiving treatments. Surgery is the mainstay of treating exstrophy for both boys and girls to improve the ability to urinate normally and improve sex organ function.